POS1275 THE HETEROGENEITY OF DEFINING SEVERITY, PROGRESSION AND OUTCOMES IN SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE: RESULTS FROM A SYSTEMATIC LITERATURE REVIEW
Authors: L. Petelytska, F. Bonomi, C. Cannistrà, E. Fiorentini, S. Peretti, S. Torracchi, P. Bernardini, C. Coccia, R. De Luca, A. Economou, J. Levani, M. Matucci-Cerinic, O. Distler, C. Bruni
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> The course of systemic sclerosis associated interstitial lung disease (SSc-ILD) is highly variable. The SSc-ILD progression (SILPRO) project aims at developing a disease specific model to define SSc-ILD progression predicting long-term, severe SSc-ILD. The evaluation of currently used definitions of severity, progression and outcomes recorded in SSc-ILD represents the highly needed first step to achieve this aim. <h3>Objectives</h3> To identify definitions of progression, severity and outcomes related to SSc-ILD in the literature. <h3>Methods</h3> A systematic literature review was conducted according to PRISMA guidelines in Medline, EMBASE and the Cochrane Library up to 31/12/2021. Eligible papers included &gt; 10 adult SSc patients, with ILD as primary target (at least one of population, exposure, outcome). Original papers in English were considered, while papers related to secondary lung involvement, ILD onset as an outcome or reviews were excluded. Two reviewers independently identified eligible studies and extracted data, including the abovementioned definitions used. <h3>Results</h3> We identified 299/8444 eligible papers, mostly reporting results of retrospective cohort studies (n=126, 42%), prospective cohort studies (n=120, 40%) and randomized clinical trials (n=24, 8%). A definition of SSc-ILD severity was included in 138 (46%) papers: it was based on high-resolution computed tomography (HRCT) data in 72 (52%) studies, on forced vital capacity (FVC) changes in 29 (21%), on combined FVC and carbon monoxide diffusing capacity (DLCO) changes in 14 (10%), on DLCO changes in 11 (8%). After combining some similar categories, 42 distinct definitions of SSc-ILD severity were identified (Figure 1 shows the most used). Sixty-one of 169 studies (36%) provided a definition of SSc-ILD "progression" referred to combined DLCO and FVC changes, 49 (29%) to FVC changes, 23 (14%) to DLCO changes, 18 (11%) to ILD extent on HRCT, 6 (4%) to combination of pulmonary function test (PFT), clinical signs and HRCT data, 5 (3%) to vital capacity decline, 5 (3%) to combination of PFT and HRCT data and 2 (1%) to other aspects (Table 1). <h3>Conclusion</h3> The studies reporting a definition of SSc-ILD "progression", "severity" and "outcome" show a large heterogeneity. These results emphasize the need for developing a standardized, consensus definition of severe SSc-ILD, to link a disease specific definition of progression as a surrogate outcome for clinical trials and clinical practice. <h3>Acknowledgements</h3> on behalf of the SILPRO project investigators. <h3>Disclosure of Interests</h3> Liubov Petelytska Grant/research support from: received research grant from Swiss National Research Foundation/Scholars at risk, Francesco Bonomi: None declared, Carlo Cannistrà: None declared, Elisa Fiorentini: None declared, Silvia Peretti: None declared, SARA Torracchi: None declared, Pamela Bernardini: None declared, Carmela Coccia: None declared, Riccardo De Luca: None declared, Alessio Economou: None declared, Juela Levani: None declared, Marco Matucci-Cerinic Speakers bureau: Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche., Consultant of: Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche., Oliver Distler Speakers bureau: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe; Novartis, Prometheus, Redxpharna, Roivant and Topadur in the area of potential treatments of scleroderma and its complications, Consultant of: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe; Novartis, Prometheus, Redxpharna, Roivant and Topadur in the area of potential treatments of scleroderma and its complications, Grant/research support from: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe; Novartis, Prometheus, Redxpharna, Roivant and Topadur in the area of potential treatments of scleroderma and its complications. Research grants: Kymera, Mitsubishi Tanabe, Cosimo Bruni Speakers bureau: Eli-Lilly, Consultant of: Boehringer Ingelheim, Grant/research support from: Gruppo Italiano Lotta alla Sclerodermia (GILS), European Scleroderma Trials and Research Group (EUSTAR), Foundation for research in Rheumatology (FOREUM), Scleroderma Clinical Trials Consortium (SCTC). Educational grants from AbbVie.